Primary Vascular-Origin Spindle Cell Sarcoma of the Cervical Region: Diagnostic and Therapeutic Challenges
摘要
Head and neck soft tissue sarcomas are rare, accounting for only 5–10% of all soft tissue sarcomas, with vascular-origin tumours being even less common. They often pose diagnostic challenges due to overlapping morphological and immunohistochemical (IHC) features. We report a 56-year-old male with a painless right-sided neck swelling for three months. Imaging revealed a large, well-defined, heterogeneously enhancing soft tissue mass in levels II–IV, compressing the internal jugular vein. Core needle biopsy with IHC confirmed a spindle cell sarcoma of vascular origin. The patient underwent wide local excision with selective neck dissection (levels II–IV), preserving major neuro-vascular structures. Final histopathology confirmed a Grade 2 unifocal spindle cell sarcoma, pT3N0, with close but negative margins. IHC was positive for Vimentin, CD31, CD34, CD99, and FLI1. The patient received adjuvant radiotherapy (60 Gy/30 fractions) due to close margins and remains under surveillance. At 12 months of follow up, the patient was clinically well with no evidence of disease. Vascular spindle cell sarcomas of the cervical region are exceptionally rare. Early diagnosis, multimodal imaging, histopathological confirmation, and a multidisciplinary approach are essential for optimal outcomes.